Posterior Atlantoaxial Fusion With C1-2 Pedicle Screw Fixation for Atlantoaxial Dislocation in Pediatric Patients With Mucopolysaccharidosis IVA (Morquio a Syndrome): A Case Series.

OBJECTIVE: To investigate the efficacy and safety of posterior atlantoaxial fusion (AAF) with C1-2 pedicle screw fixation for atlantoaxial dislocation (AAD) in pediatric patients with mucopolysaccharidosis IVA (MPS IVA). METHODS: This study included 21 pediatric patients with MPS IVA who underwent posterior AAF with C1-2 pedicle screw fixation. Anatomical parameters of the C1 and C2 pedicle were measured on preoperative computed tomography (CT). The American Spinal Injury Association (ASIA) scale was used to evaluate the neurological status. The fusion and accuracy of pedicle screw was assessed on postoperative CT. Demographic, radiation dose, bone density, surgical, and clinical data were recorded. RESULTS: Patients reviewed included 21 patients younger than 16 years with an average age of 7.4 ± 4.2 years and an average of 20.9 ± 7.7 months follow-up. Fixation of 83 C1 and C2 pedicle screws was performed successfully and 96.3% of them were identified as being safe. One patient developed postoperative transient disturbance of consciousness and one developed fetal airway obstruction and died about 1 month after the surgery. Out of the remaining20 patients, fusion was achieved, symptoms were improved, and no other serious surgical complications were observed at the latest follow-up. CONCLUSIONS: Posterior AAF with C1-2 pedicle screw fixation is effective and safe for AAD in pediatric patients with MPS IVA. However, the procedure is technically demanding and should be performed by experienced surgeons with strict multidisciplinary consultations.

Single-Stage Posterior Approach for Multilevel Cervical Ossification of the Posterior Longitudinal Ligament With K-line (-) Using Thick Cervical Pedicle Screw System: A Technical Note and Preliminary Results.

STUDY DESIGN: Technical note, retrospective case series. OBJECTIVE: The optimal surgical strategy for multilevel cervical ossification of the posterior longitudinal ligament (OPLL) with a negative kyphosis line (K-line (-)) remains controversial. We present a novel single-stage posterior approach that converts the K-line from negative to positive in patients with multilevel cervical OPLL, using a posterior thick cervical pedicle screw (CPS) system and report the procedure's outcomes and feasibility. METHODS: Twelve consecutive patients with multilevel cervical OPLL and K-line (-) underwent single-stage posterior thick CPS fixation, with laminectomy and foraminal decompression. A pre-bent rod was installed to convert the K-line from negative to positive. Radiographic parameters, including the extent and occupying ratio of OPLL and the C2-C7 angle, were examined. CPS accuracy was assessed using computed tomography. The Japanese Orthopaedic Association (JOA) and visual analog scale (VAS) scores were analyzed. Quality of life was assessed using the Neck Disability Index (NDI). The mean OPLL extent was 5 vertebral body levels, and posterior decompression was performed on 4.2 segments. RESULTS: The average C2-C7 angle and the occupying ratio of OPLL improved from -9.0° to 14.3° and from 63% to 33%, respectively. The preoperative JOA, VAS, and NDI scores significantly improved from 8.4 to 13.3, from 7.1 to 2.2, and from 21.9 to 9.3, respectively. The K-line was converted from negative to positive in all cases. No severe complications were identified. CONCLUSION: Single-stage posterior surgery with a thick CPS system may be a reliable and effective treatment for multilevel cervical OPLL and K-line (-).

Naringin Inhibits Apoptosis Induced by Cyclic Stretch in Rat Annular Cells and Partially Attenuates Disc Degeneration by Inhibiting the ROS/NF-κB Pathway.

Oxidative stress and apoptosis play important roles in the pathogenesis of various degenerative diseases. Previous studies have shown that naringin can exert therapeutic effects in multiple degenerative diseases by resisting oxidative stress and inhibiting apoptosis. Although naringin is effective in treating degenerative disc disease, the underlying mechanism remains unclear. This study is aimed at investigating the effects of naringin on oxidative stress, apoptosis, and intervertebral disc degeneration (IVDD) induced by cyclic stretch and the underlying mechanisms in vitro and in vivo. Abnormal cyclic stretch was applied to rat annulus fibrosus cells, which were then treated with naringin, to observe the effects of naringin on apoptosis, oxidative stress, mitochondrial function, and the nuclear factor- (NF-) κB signaling pathway. Subsequently, a rat model of IVDD induced by dynamic and static imbalance was established to evaluate the effects of naringin on the degree of degeneration (using imaging and histology), apoptosis, and oxidative stress in the serum and the intervertebral disc. Naringin inhibited the cyclic stretch-induced apoptosis of annulus fibrosus cells, reduced oxidative stress, improved mitochondrial function, enhanced the antioxidant capacity, and suppressed the activation of the NF-κB signaling pathway. Additionally, it reduced the degree of IVDD (evaluated using magnetic resonance imaging) and the level of oxidative stress and inhibited apoptosis and p-P65 expression in the intervertebral discs of rats. Thus, naringin can inhibit cyclic stretch-induced apoptosis and delay IVDD, and the underlying mechanism may be related to the inhibition of oxidative stress and activation of the NF-κB signaling pathway. Naringin may be an effective drug for treating degenerative disc disease.

Cervical spine involvement in pediatric mucopolysaccharidosis patients: Clinical features, early diagnosis, and surgical management.

Mucopolysaccharidosis (MPS) is a progressive genetic disease that causes a deficiency in lysosomal enzymes, which play an important role in the degradation pathway of glycosaminoglycans. As a result of enzyme defects, mucopolysaccharides cannot be metabolized and thus accumulate. The cervical spine is one of the most commonly involved sites; thus, prompt surgical management before the onset of severe neurological deterioration is critical. However, because of the rarity of the disease, there is no standard treatment. In this review, we characterize the cervical spinal involvement in pediatric patients with MPS, describe the useful imaging technologies for diagnosis, and provide screening procedure for children with MPS. Surgical managements, including indications, surgical methods, possible difficulties, and solutions, are reviewed in detail.

LncRNA LINC00588 Suppresses the Progression of Osteosarcoma by Acting as a ceRNA for miRNA-1972.

Long non-coding RNAs (lncRNAs) are being found to play an increasingly important role in the development of tumors. However, their biological functions and the underlying mechanisms remain unclear. Using information from GEO Datasets, we found that the lncRNA LINC00588 was downregulated in osteosarcoma (OS) in bone but was upregulated in the metastatic tumor present in the lung. We assessed the function of LINC00588 using both overexpression and knock-out studies. We performed colony formation assay, CCK-8 assay, flow cytometry, wound healing assay, transwell assay, and RT-qPCR assay and used a xenograft model to investigate the influence of LINC00588 on cell proliferation, viability, cell apoptosis and cycle, migration, invasion, endothelial cell function, EMT (epithelial to mesenchymal transition), and tumor growth, respectively. Overexpression of LINC00588 appeared to inhibit cell proliferation, viability, migration, invasion, endothelial cell function, EMT, and tumor growth but not apoptosis, while we got the opposite result when we knocked down LINC00588. Next, we predicted that LINC00588 bound to miRNA-1972 and significantly downregulated its expression, which we then verified through a luciferase reporter assay. Subsequently, we knocked down miR1972 and performed CCK-8 and transwell assays to demonstrate that downregulation of miRNA-1972 could substantially inhibit the viability and invasion of osteosarcoma cells. The expression of TP53 was downregulated at the protein level but not at the mRNA level after the overexpression of miRNA-1972. Taken together, our findings indicate that LINC00588 plays a role in OS development by downregulating the expression of miRNA-1972, which can, in turn, inhibit the expression of TP53. Hence, we believe that the LINC00588/miRNA-1072/TP53 axis could potentially serve as a therapeutic target or diagnostic biomarker for osteosarcoma.

Efficacy and Safety of Atlantoaxial Fluoroscopy-guided Pedicle Screw Fixation in Patients Younger Than 12 Years: A Radiographic and Clinical Assessment.

STUDY DESIGN: A retrospective clinical study. OBJECTIVE: The aim of this study was to evaluate the efficacy and safety of fluoroscopy-guided atlantoaxial pedicle screw fixation in patients younger than 12 years. SUMMARY OF BACKGROUND DATA: C1-C2 pedicle screw fixation is a widely accepted treatment method for atlantoaxial dislocation (AAD). However, data regarding its use for atlantoaxial fusion (AAF) in children are limited. METHODS: Thirty-six consecutive patients younger than 12 years underwent C1-C2 pedicle screw fixation for AAD between 2007 and 2017. Anatomical parameters of the C1 pedicle were measured on preoperative computed tomography (CT). Accuracy of pedicle screw fixation was assessed on postoperative CT using the following definitions: Type I, screw threads completely within the bone; Type II, less than half the diameter of the screw violating the surrounding cortex; and Type III, clear violation of the transverse foramen or spinal canal. Demographic, surgical, radiation dose, and clinical data were recorded. RESULTS: Patients underwent 144 screw fixations (67 C1 pedicle screws, 68 C2 pedicle screws, 5 C1 lateral mass screws, and 4 C-2 laminar screws) for a variety of pediatric AADs, with 36.5 ±â€Š8.5 months of follow-up. Among the 135 pedicle screws, 96.3% were deemed "safe" (Type I or II) and 80.7% (109/135) of the screws were rated as being ideal (Type I); five screws (3.7%) were identified as unacceptable (Type III). Average estimated blood loss (EBL) was 92 mL, and the average total radiation exposure during the operation was 6.2 mGy (in the final 26 cases). There were no neurovascular injuries. All patients showed radiographic stability and symptom resolution. CONCLUSION: C1-C2 pedicle screw fixation under fluoroscopy is safe and effective for the treatment of AAD in children younger than 12 years. However, it may be technically challenging owing to the special anatomical features of children and should be performed by experienced surgeons. LEVEL OF EVIDENCE: 3.